Non-typical persistent hyperplastic primary vitreous: a rare case report and review of the literature

BMC Ophthalmology

Case Report

Jianghua Hu¹  Ke Yao²  Yinhui Yu²  Yue Qiao²  Silong Chen²  Jinyu Li²  Yibo Yu² 

[1] Department of Eye Center, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China;Department of Ophthalmology, Jiande Branch, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China;Department of Eye Center, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China;Zhejiang Provincial Key Lab of Ophthalmology, Hangzhou, Zhejiang Province, China;

关键词: Persistent hyperplastic primary vitreous;    Age-related cataract;    Diagnosis;    Histopathology;    Surgery;   

DOI  :  10.1186/s12886-023-03024-x

 received in 2022-11-03, accepted in 2023-06-06,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundPersistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature (PFV), is a clinical entity that traditionally presents with leukocoria, microphthalmia, retinal dysplasia, or eyeball shrinkage which is associated with poor vision. However, there is a dearth of literature on cases of PHPV in adulthood or with asymptomatic occurrence. This report presents the clinical and pathological findings of a non-typical PHPV case and discuss the current knowledge for this condition.Case presentationA 68-year-old healthy male was referred to our outpatient department for evaluation of age-related cataract without other visual symptoms. Preoperative fundus examination occasionally detected an isolated stalk-like band extending to the posterior pole of the eye with normal central vitreous and retina. Other ocular examinations including b-mode ultrasonography, optical coherence tomography did not unveil any abnormalities, which caused diagnostic uncertainty. We referred to cataract surgery along with histopathological study, that revealed characteristics of PHPV including fibrous connective tissues mainly composed of fibrocyte proliferation and a very few capillary vessels. Thereafter, a definitive diagnosis of non-typical PHPV was established.ConclusionOur case is unique due to it was not discovered until adulthood, presence with only age-related cataract, and accompanied with normal central vitreous and retina. Histopathological explorations lead to an accurate diagnosis of the condition. Those results broaden the phenotype spectrums of PHPV and further provide clinical clues for the cognition of the disease.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

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Fig. 2